A catch up post

Well, we finally seem to be recovering from a hectic and stressful week last week. The house is finally clean and tidy again after all the celebrations last week Mum and Dad were here from Stoke-on-Trent Tuesday and Wednesday. One purpose of the visit was to collect the girls if Wills had gone into GOSH but they were also here to see us all following Hope's birthday and to visit CHASE for a Grandparent's session. It has been lovely to have them for a few days and actually relax with them rather than me being in hospital with Wills and them picking up the pieces with the girls. Paul has now begun Glyndeborne performances so will not be around in the evenings much until September, then briefly before going on tour so it was nice to have a busy house for the last few days.

William had his 2 year developmental check today. He had to see a member of the community paediatric team due to his "issues". It turned out to be really useful as she as able to hurry the physio appointment (thanks to Wills walking so badly she commented - "he really is trying..." - he runs at home, I told her, all be it in his unique wobbly style but he does it!). She has also referred him to a communiy paediatrician in the children with disability team whose role will be to co-ordinate with the many people involved in him and pull it all together, refer him to others where needed etc. That is great because we really need that. His feet were a cause of some concern as his arch and instep are now so high they were described as "deformed". If that is the case, they will, hopefully be corrected by boots and/or splints. She was delighted by his wonderful, social personality though - everyon who meets Wills loves him for that. I keep saying it, but I have never met a happier, more socialble little toddler - Hope and Ellie would agree with me too. After that, we had a brief coffee with Grandma and Grandad and said goodbye before popping home to take the TPN down and off to visit the Opportunity Playgroup for children with special needs. This was lovely and I am looking forward to William starting when a place comes up. He will have speah, physio and occupational therapy there. I do have to stay though as the staff are unable to so the medical procedures he needs. Another busy and hectic day!



A sad part of today was saying goodbye to our very best friends round here. The photo above shows us all together for a barbeque a few weeks ago (I, of course am taking the pics as usual). Clare is William's Godmother and has been an amazing support for us, having the girls over and over again when he has been suddenly taken ill. One of her daughters has been Ellie's total best friend from the first day she walked into school. We will all miss them so much but are looking forward to visiting them in Somerset. A great moment though was that one of the community nurses who looked after him when he was on oxygen, for weekly sleep studies etc, and who had left the team, found our number today and got in touch. We were becoming good friends and now there is no professional distance to be maintained I am really looking forward to developing our friendship. We are visiting her Monday and I can't wait! It has been a year since we last saw her, just as William was being diagnosed with "presumed CF" and she was so great through it all. It took me ages to describe how we got from there to where we are with William now!

Ellie's turn for centre stage!

Well, the a "sleep over" was the wrong word for what went on in Hope's room last night. There was very little sleep! The girls had such a great time and it really seemed to go down as a coming of age event! I was mighty pleased to be told I was "a really cool Mum" by one of Hope's friends. I'm not sure I was so cool when I went into their room at 1.30am and about 3am to tell them to be quiet! Most of the time they were and I did turn a blind eye to the fact they were on a pact to stay awake all night - we've all been there! It was only when they woke me that I reminded them of the others in the house - gently, I didn't want to spoile their special night as it was their first sleep over. There was make-up, chocolate, hair stuff and girliness in every inch of the room. You should see it today!

After a continental breakfast and the mundane task of supermarket shop to fill our fridge now we are not off to GOSH, the attention switched to Ellie. I was rather concerned as 2 parents phoned to say their girls had stomach bugs and couldn't come. I imagined them dropping like flies and a low turnout - maybe they have been eating too many Cadbury's chocolate ;-). It gor pretty hot so we opted for team games on paper like pictionary and drawing monsters and telling stories by forlding the page so no-one can see etc. It went down really well.




You may notice the absence of Hope on these pictures - she was totally crashed out on the sofa! It was a great afternoon. Well, the house is now trashed so I am mighty relieved I don't have to finish all my work and pack for GOSH tomorrow!

One of those days big time and no GOSH for now!

As I write, there are 4 very excited and happy girls upstairs telling each other ghost stories and discussing the boys in their class (and this is the 10th birthday - what is to come???)


It has been one of those days! We had clinc at 11 so I decided to go straight from school pick up, rather than pop home for about half an hour, and get a coffee when we got there. The trams decided to come to a complete standstill at Mitcham so we had to get a slow train to Victoria and then a very slow bus. I could have done that much quicker from East Croydon! So, we were late for, what was a very manic clinic. Still, we should have been seen by about 12.30 as they were running an hour late. We finally went in at 1.20 as several nurses who don't usually work there kept putting later appointment's notes on top of Williams and some parents were even switching them. We had a good old chat with the consultant though who is also beginning to feel that William's gut problems are syndrome related and agreed that he is very characteristic of Costello Syndrome. I am not going to go into any more about this syndrome yet. This time last year we were told it likely William had CF and told to read up and get involved etc. I have read a bit about Costello and agree it fits Wills very well but am not getting too involved until he has a clinical diagnosis at least. They are going to talk to the geneticists about it and I will post more if they agree. We also discussed the pros and cons of the illeostomy. It was nice because he shared that it had been a really mad clinic and was pleased to be able to relax with our appointment. It is nice when you get that rapport with your consultant. He stressed the need to get our local paediatrician more involved in overseeing William's increasingly complex and varied issues. Everyone agreed that his feet and walking are becoming an increasing problem and it could be that this, and the gastro problems, are neurological due to the syndrome (whatever it may turn out to be).

Anyway, I flushed Wills off the TPN and was set to rush and get the girls from school as time was now well and truely racing by. I got to the bus stop only to find I had left my handbag with absolutely everything in it! Needless to say, it wasn't there and no-one at the clinic had seen it. I couldn't believe it, I had had it 5 minutes before! Eventually, much to my relief, it turned up at security. By then, I was really getting late for the girls. The tram driver at Wimbledon watched me run passed him and pulled away as I reached the door (something Tramlink drivers are only too good at doing!). I was 30 minutes late for the girls.

Later, Hope went up to the shops for bread. While she was gone, I chased GOSH to be told that the child who was going home is not anymore and William will not be going in next week and may do the week after but then may not for weeks. It was such a comedown as I had been so high on adrenaline getting ready to go. I do feel for the family that thought they were going home though and understand how difficult it is to predict these things but it is hard to have a plan for the girls that is then not needed and just be expected to come up with another one in a few days or few weeks time! It is a rollercoaster, it really is. Anyway, as all this was going on, Hope came home and I even opened the door for her. 20 minutes later, I was getting worried that she hadn't got back so went looking for her, along with a neighbour I met on the way. I couldn't see her and came home thinking she would be there but didn's see her outside. So, getting seriously worried, I came in to think what to do next with the neighbour still with me -only to find she had been home all along and I had let her in! It had been a long day.

All the girls sleeping over called to make sure it was still on in case of a William crisis. One Mum, a good friend of mine, brought round a bottle of wine as I sounded so stressed - bless her. It has now been consumed and I am much calmer. The sleepover has been a huge success. Ellie did not get the predicted strop about being left out and is still having her party tomorrow as life is very much in the air and unpredictable at the moment (GOSH could still ring Monday and say later this week!). Mind you, it always has the potential to be like this. We really do live one day at a time these days.

GOSH on Monday!!!

A couple more photos from Little Chaser's last week - William very grown up on the train and another from the music session.

Well, we got the call. William's bed at GOSH should be available for him on Monday! Much sooner than we expected and I have very mixed thoughts about it. My head is saying it is good that we are going so soon as we can't move forward until we get these last tests done. However, my heart is struggling with the thought of being seperated from the girls once again and feeling so isolated in hospital with William in the evening when he is asleep. Paul will be so busy at Glyndeborne and won't get back until midnight once the shows kick in so we won't see a great deal of him. I hope this stay won't be long but we have been booked into a long stay bed. I am also nervous of what they may come up with as a diagnosis. The community nurses today agreed that his walking is getting worse and his balance and gross motor control is clumsy and unsteady. I just hope that there is no brain involvement. He clearly has "syndrome features" I can see that (although I must say they suit him very well and he is beautiful!) and so many syndromes have brain involvement. I guess, we fear that his walking and balance is worsening so where could that lead? Of course, it may only be that he is deficient in some mineral in the TPN and correcting that could see him walking tightropes! All will soon become clearer and that is great, but scarey too. So far, everytime we have been in hospital for tests new things have been found and the picture has become more complicated. However, there does seem to be a cluster of syndromes that he does fit so, hopefully, things will get clearer soon. Once we know what we are up against we will be able to plan and ensure he reaches his potential in every way and has the best life possible. Then, of course, there is the issue of the transplant assessment that may come soon after this hospital stay.

It's a good job I moved Ellie's party to Saturday. We will, at least, get the girl's birthday parties in before Monday. Hope's birthday is tomorrow so I must get wrapping...

Great Ormond Steet - At last!

Well, we finally heard from GOSH this morning to say that William will be admitted within the next few weeks. Apparently, they plan to have him in "long term" to assess his clinical picture etc before planning their tests. I have heard that GOSH tend to disregard everyone elses tests and start from scratch! Well, there are still a lot of unanswered questions so a new pair of eyes over everything may not be too bad a thing. There is more scope for multi-disciplinary discussion about his lungs, minor heart defect, co-ordination and walking and "syndrome facial features" etc in addition to his main, intestinal problems. I hope that long term does not mean too long. It doesn't look like we will get much warning when the bed comes available so we are now going to have to make sure we are always only a couple of days away from being able to pack up life and send the girls to Grandparents and Wills and me into the hospital. This will mean staying on top of washing etc and making sure I have all the stuff I need to carrry on with work etc. I will also be assembling some treats like a few good books, DVDs etc as the televisions on the patient line will, no doubt, go off at 9! C and W have manged to negotiate all night TV for long term parents which is so great! Paul will be really busy with Glyndeborne so there won't be home until midnight most of the time so it is going to be a bit lonely. In the meantime, ellie's birthday party has now been bought forward to next weekend to make sure we get it in. Hope has hers on Friday anyway so it will be a weekend of it! Poor Ellie, This will be the 3rd time she has had William and hospital overtaking her birthday - he was born the day after her birthday so I was in hospital then, last year he was in the Brompton for it, as well as his first birthday, and this year looks almost set to be another year where he will be in hospital for both Ellie's and his own birthday. Poor Wills has not yet had a birthday or Christmas uninterrupted by hospital! Of course, this isn't too bad for him, in fact he probably enjoys the extra attention, but is tough on the girls - and me. Who knows, we may be out by then but Ellie's party was planned for before the end of term, otherwise, everyone goes away, so we definately need to get that in quickly. Of course, we were told "within weeks" back in March but this time it is in writing. We could still be waiting at Christmas though!!!

Little Chasers

We had a lovely time yesterday at "Little Chaser's" pre-school group at CHASE Christopher's Hospice. It was a music therapy session and William was totally captivated! He couldn't keep his eyes off the leader and smiled and smiled, joining in with the singing and playing instruments as best he could. He was most cross to be dragged out for a while to be assessed by the physio. The verdict was that he does have a tendency to walk on his toes but that could be a habit. However, he seems to have a slightly weaker left side and his tendons are a little tighter. This makes him pick up and bend his leg more on the right, resulting in a bit of a "drunken swagger" leading to lots of falls. His feet are also a bit too pointy with a big instep - more like adult than childrens feet. This would not be too much of a concern apart from the fact it is getting worse rather than better. She is writing a report to our local paediatrician and physis as it needs further investigation. Naturally, we really hope that he isn't going to have a physical disability. He is so determined though and still runs and runs, even though he swaggers into things and falls flat on his face so much at the moment. He reminds us of the song "I get knocked down, but I get up again, you'll never going to keep me down..." He falls and struggles so much more than the girls did yet only cried for a few seconds - such a little fighter!

Ellie is back tomorrow from her school journey to an activity centre. It has been very quiet as Hope has had no-one to argue with but we have missed her loads. William had a respite nurse in on Tuesday so, with Ellie away, Hope had a very rare couple of hours after school alone with Mummy! We spent the time shopping for her "girlie pampering sleep over" she is having next Friday for her birthday. It will be her first sleep over - please stay well Wills!

Walking on Tippitoes

Poor Wills is really struggling with the heat and spiking temperatures very close to our magic 38 degrees at which he has to go into hospital! His nappies are drying up but we are still waiting on the TPN company to see if they can get a stable bag of TPN with even more fluid than the extra he already has to replace his gastrostomy losses. So far, they have not been able to make the solution with extra fluid stable. He is such a sweaty little thing. Still he smiles though!!!

The really worrying thing this morning is his walking. He is walking up on his toes most of the time and wobbles and falls over all the time. This has been getting worse but neither Paul or I have wanted to voice it for fear of making another problem real! However, he now has now been referred for a physio assessment. He seems to have very tight tendons that force him on his toes and his instep is getting so high we struggle to get shoes to fit. He may need to have special little boots to correct it. The question is why though? It may be that this is another feature of a "syndrome". I would be so sad if it turned out that he has some physical disability as well as his medical symptoms. Mind you, William is one little boy who will not let anything stop him. OK, he falls over a lot but he very rarely cries and just picks himself up and gets on with it! He can go very fast on those little toes too!!!

Come on England!

We are just watching "The" match so thought we should share this picture of William with John Terry at Christmas last year, together with "Terry" the cuddly dog he gave William for Christmas. The Chelsea team came in with presents for the children as they are just up the road. it was very exciting for them all (well, the older ones amnyway!). Sadly, our close friends had lost their little Riley in the early hours of the morning, having just got home after nearly a year in hospital so spirits were a bit down among the gastro team and parents. Riley was to be a year old in just 3 days time on Christmas Day! Still, the arrival of the team helped us get back some Christmas spirit and when the day came we all drank a toast to "Smiley Riley!"

Anyway, the purpose of this post was not to think about this but to share with you the picture of Wills with John Terry (although, the memory of Riley will always go with the happy memories of the day). William was lucky to get the captain himself giving him his toy - although the players did seem a little overwhelmed to see a baby on so many drip pumps he needed two drip stands! It was exciting to meet the team and we are cheering them on today with the other England players. I am looking forward to showing William his autographs and pictures in the future - especially if England win this year and the names of some of those he met go down in history - come on England!!!

A wonderful day at Legoland

We have had such a wonderful couple of days it really feels like we have had a weeks holiday. The zoo day was a real treat and then on Sunday we went to Christopher's Hospice for an overnight stay before Legoland on Monday. We didn't get William's swim as the pool was out of action for 24 months, following a "little accident" the day before and then the chemicals were wrong. We will get him in the pool eventually! As you will see later, he did get to use his suit again though. Instead, WIlliam enjoyed the trampoline with his sisters and was really getting confident. He love sitting and being bounced and even ran across it - much to everyone's amusement as he couldn't stop when he got off!
Once again, we were really chilled out in a couple of hours. This time, I got a complete break from all William's care as we have completed the care plans. It was hard to let go but I was determined and by the time we had finished tea William's TPN was up and running and he was even asleep before I could say goodnight! This was a big step towards, eventually, leaving him there for a night to go out and have some time with Paul and some friends.

Legoland was an amazing day. There were two families and we had clearly been very carefully matched for ages of the children and personalities etc. We all got on really well and had a lot of fun, as well as lots of heart-to-hearts between us Mums. We had made plans to meet up at the City Farm that is half way between us before lunch time! Ellie went round holding hands all day with her new friend and Hope and the eldest son were equally mature and serious about everything and engaged in amusing, friendly competitiveness on the rides that had a race element. Even Willliam had some similarities with his new little friend as there was some overlap in their conditions, especially regarding gut motility and absorption etc. She is not on TPN but nearly was at one point. This similarity led to an interesting insight - she is needing the same test we are waiting for at GOSH but, being a GOSH patient, knows the inside info - the machine (the only one in the country) is currently out of action and awaiting a part from Norway which no-one expects to get for months!!! If only we had been told that reason for the delay. William is still in increasing pain everyday so we need to discuss this situation when our consultant is back as it is unfair to keep him suffering indefinately as this test was supposed to be done prior to an illeostomy.

William loved Legoland, they all did. There is a lot for young children. He loved the model London, especially his beloved trains and buses! He couldn't wait to get off his TPN and start climbing in "Duploland" and have a ride on a lego train.

We went on fire engines, hot air ballooons, monorails, trains...the girls took their driving test and were treated to their license. The children had a really great time and were so happy all day. They felt very special going to the front with their exit passes as 3 people could go with each "disabled" person.

We all went on a very exciting water splash ride. Luckily they still let us get to the front although the two little ones with the "red stamps" clearly couldn't go on. They got their chance to get wet later.



William got to use his suit to discover the water jets and activities in the "Kids Wash". He could't have gone in their without the suit as he got dripping wet. He was scared at first as this is all so new to him and I did get some looks as he was screaming. He does for a while and then really enjoys the water. I'm sure some people thought it hugely over the top as he was in a dry suit!



It was an amazing day and we made some new friends which is great for all of us as the children now have some friends who are in the same boat as them. This was one of the main purposes behind the day - to give the children and Mums some fun and happy memories of a great day out and also to help families get together and form supportive friendships. CHASE has a motto "Just because a child's time may be limited it doesn't mean their life should be" The last few days have certainly been so for us, thanks to them! Both of us Mums were saying yesterday what a huge difference top our lives CHASE has made. She is now planning to do the Hydro-active as well for CHASE. Thanks to those who have sponsored me already - these photos today show where that money goes in making time-limited children have full lives that are anything but limited and helping families enjoy the time they have together. There is more about CHASE on my justgiving page which is linked on this blog, under the profile with the links (I can't do flashy links in the text but may get Paul to do it for me later!) We nearly had tp be blue lighted out though as there was an accident on the motorway, resulting in static traffic right into the carpark! William's TPN was due up and was already going to be an hour late once we had waited to see what was going on. Luckily we were shown the staff way out - an interesting tour of the backstage of Legoland.

The children were even giving spending money and, as I am writing, William is busy with his duplo that we added to with his, racing a duplo train Mummy helped him to make with his Bertie Bus. it is keeping him very happy in his pen on his TPN.

Wisley Gardens and Marwell Zoo

We had a lovely day yesterday. I love this picture of all 3 children having such a lovely time together at Wisley Gardens. They were so happy and it is the first time we have had a really long day out together since William was born really. We set off early and got to William's Granny's house in time to get William off his TPN - we had worked it forward so he could be put on before going to the zoo in the evening. His usual time would have been in the middle of the journey from Christopher's Hospice to the zoo. We flushed William off and he toddled and toddled in the garden while we all had an early lunch. We then set of for Wisley Gardens.

William loved feeding the ducks but was much more interested in the huge fish that were swimming so close to his feet...so clost that he wanted to climb in and join them

At least it looks like he isn't going to be scared of the water! (We were hoping to go swimming at the hospice tomorrow but they called earlier to say there was a little accident in the pool this afternoon and it needs to be shut for 24 hours!)

After a few hours at Wisley we were off the Christopher's for some tea and to get William set up on his TPN and then off to the zoo. It was quite a drive and we were in convoy with other families in their cars and loads of taxis. We nearly ran out of petrol as we were depending on a station in the last bit when we couild easily find the zoo - but there just wasn't one! Luckily the taxi drivers found us one just a bit further on with their GPS systems. William was absolutely fascinated with every animal. This picture below shows him wide eyed in the giraffe house - they were being fed and were just feet away. He was amazed! His little face was much the same looking at every animal.

We saw so many animals and William really was interested in all of them. It was a great evening. The zoo held a special, free opening for children from hospices, special schools and residential homes etc and treated them to the animals together with special talks and demonstrations, craft activities, characters wondering around, goodie bags and, apart from the animals, best of all for William miniture trains, tractors and...a visit from the local fire brigade and police. This was William's reaction to the fire engine...

He loved it so much that he just had to climb in and have a go!!!

All in all it was a fantastic day and we were so amazed at how Marwell Zoo did all this for these children and their families. Tomorrow, we are off to Christopher's and may get the chance to swim with William for the first time (if it is not too busy there is a brief slot between the 24 hours "decontamination" and William going back on TPN. Then on Monday, we are off to Legoland with CHASE. The only blot on a perfect time is that poor Wills is in a lot of pain at the moment and really suffering and screaming and crying - still smiling in between though!!!

A quiet couple of days

Well, I think I am finally over the trip to Kings. I was so exhausted and achey all day yesterday and ended up in bed by 9! The girls' and I watched Bambi with our big bowl of popcorn yesterday afternoon. I have never actually seen it so it was nice to finally do do (although very sad!) We are all spending a lot of time re-reading Harry Potter at the moment. Hope, Ellie, Paul and I are all at it. I am on the last one and Paul the one before which is going to get very nasty soon as Paul spends about 3 hours a day on the train at the moment!! William enjoyed his Wiggle and Jiggle today but has had a really uncomfortable day with his tummy. His especially thick yukky aspirate is back, complete with the big "coffee granules" and he is "pooling" his output too which means it all hangs about and builds up in his system, resulting eventually in a huge, windy explosion which goes absolutely everywhere. Unfortunately, this causes loads of discomfort and pain. His tummy is hugely distended and his breath even smells so there is a lot building up today. Once he "explodes" we get a few days of constant output. I am really looking forward to him having an ileostomy now as it will make him so much more comfortable. I thought I'd never say that when the suggestion he may need one was first made. He is still smiling though - as always. When he was feeling it a bit today he looked at me and said "oh, Mummy, oh dear" with big eyes in the way children ask you to make it all better with a believe that you can. It was the first time he has done this as his language is improving. It was sweet but heartbreaking as there is noting I can do except massage him a bit and distract him with a favourite book.

Hopefully he will have a good day tomorrow because we are off to the zoo with CHASE. It is going to be a lovely day as we are having an early lunch at William's Granny's before a few hours running around at Wisley Gardens and then to Christopher's Hospice for an early tea at 4 and on to the zoo. William has never been before and, since the trip to the farm on Monday, has been animal mad. He has been looking at a picture book about a lion today and roaring at it so I think he is going to get really excited tomorrow. I only hope he stays awake as it is a special evening opening just for CHASE from 6-9! we should have some lovely pictures to put up here tomorrow night - hopefully of three excited children, not two excited girls and a sleeping toddler!

An exhausting day!

Our trip to Kings College Hospital today turned out to be a totally exhausting experience!!! It was a horrible place to get to involving train changes with huge staircases and no lifts! I had to carry William, the pushchair, his pump and bags up so many stairs I could have cried by the end of it all. People did help out a couple of times but on one huge long staircase at Denmark Hill there was a girl behind me having to wait (this was a narrower one with no passing space) while I paused and got my breath back and rearranged William and never once offered to help. On the way home I was desperate for a coffee and stood at the top of the stairs with William and my coffee and asked the next person who passed to carry my coffee down. It was such a long day.

William's ultrasound showed that his liver is enlarged and fatty - but we knew that anyway. They took loads of bloods (26mls worth so I dread to think what his Hb is now!!) and will look at those together with his ultrasound to make sure there is nothing else going on other than the effect of TPN. As long as his liver numbers are OKish and he is doing OK on the TPN, they feel it will be better for him not to go for transplant as it will enable advances to be made in bowel transplants (they are still very new and behing other organs) and the bigger he is when he needs it the more chance he will have to get an organ. However, once it is confirmed that he is definately on TPN until a transplant and no hope for any other cure it will be a fine balance to make between leaving it as long as possible and too long. If we wait until liver failure there is always a chance a donor will come too late whereas if we go too soon his life could have been lengthened by waiting... At least we are not at that stage yet. They explained that infections will speed up liver failure - we have already seen this as his liver gets cross and his liver function tests go a bit off when he has infections and he tends to never completely recover back to where he was before. So, it is even more important that we get to the bottom of his current pattern of repeat chest infections - even if it means a repeat Nissens. They are just checking he hasn't got any other form of liver disease but, otherwise, they will be kept informed by Chelsea and Westminster of how things are progressing and will see him again when (and they did say it is a when) his liver function tests go really bad. Today was really about getting a baseline I guess.

We had planned a day out tomorrow but today was so tiring and with such excitment to come later in the week we are going to pace ourselves (well me really) and have a day in. I am thinking a good old girly film Hope, Ellie and I will enjoy when William is asleep and a huge bowl of popcorn!

Godstone Farm

Today we were determined to fit a family day out into our 6 hours off TPN. It was one of those very rare occasions we were all free too! There is a fantastic chidren's farm that we found out was close enough to get there and back and still enjoy a good 3 hours fun. William had his first real close encounter with a rabbit which he was really fascinated in. He was pretty gentle too and the little bunny was definately used to toddlers stroking a little too close to the eyes. He also loved the "cows" and "neighs" and "baas" but best of all was the tractor! Yes, the genetic little boy obsession with all things vehicle seems to have been well and truely switched on at late. On Saturday, his Godmother sent him a little "Bertie Bus" who has been racing "Thomas the Tank Engine" all over the house ever since. William kept running back to his beloved "tata". In fact, he ran everywhere, even up and down some very steep banks on the nature walk and woodland maze. He really does pack his whole day into these 6 precious hours! We also enjoyed the vast stretch of adventure playground where the girls loved the zip wire and William had a fantastic time on the swings and in the sandpit. We even found the time for a cup of coffee and huge slap of cake while all 3 children sat in a very intense pow-wow on the grasss looking very deep in discussion. I was gutted that my camera was out of battery at that point as I forgot to charge it this morning.

On Friday we are off to a special evening opening at the zoo with all the other CHASE families. That will be great and Paul's Glyndebourne rehearsal has been moved from that day so another rare opportunity for a special family time - two in one week! William is just about ready to get a lot from it too as he is really showing an interest in seeing all the animals now. He has come on so far from just a couple of months ago when I first took him to our closest city farm and he really didn't pay a huge amount of attention. Now he just keeps going back to say "bye bye" to everything and watch some more (bye bye being his word for hello and goodbye!).

Tomorrow, we are off to Kings Hospital for William's liver scan and to see his liver consultant. By coincidence, his bilirubin today is the highest it has been in months - still OK at the moment but I don't like upward trends. A good time for his Kings appointment though as they will, hopefully, be able to nip anything in the bud.

Clinic

Another clinic this morning. The first question was about how William is in himself now. Luckily, he is pretty good today or I get the feeling we would have been admitted. We have to bring him in if there is any more blood so he can have an emergency colonoscopy and endoscopy. Following the disastrous result of having oral antibiotics he is now not to have any more oral meds, apart from the Urso and Vitamin K as they are such tiny quantities. We do doubt they are actually being absorbed though. He has to go to Kings for a liver work-up on Tuesday so we may address what to do about them there. They will also look at his fatty liver and the fact his liver enzymes are up a bit at the moment. W discussed his chestiness and recurrent nasty chest infections and pneumonia today. We have to get him reviewed again at the Brompton but, if it continues, he will have another endoscopy to see if he is pooling saliva and stuff in the upper GI tract, ready to aspirate and choke on. It may also be that his Nissens is coming undone and may need repeating. Poor thing, so many operations and procedures. So, all in all, we are muddling along but not quite as well as we were. Hopefully this is just because he has been unwell and it will all pick up again. It is a shame the antibiotic had such a huge impact as it looks more and more like William just can't be fed, at least not for the foreseeable future. Unless there are any emergencies over the next 4 weeks, all this will be re-assessed then as his consultant is on holiday. When he gets back, we will have to see if William needs the endoscopy and if he needs yet more fluid in his TPN to make up for the huge losses he is having in his stools and gastrostomy drainage at the moment.

Wiggle and Jiggle and Splish Splash Splosh!

Well, after a bit of an anxious evening, neither Paul or I wanted to check the morning nappy for fear of what we might find. Paul eventally wondered in (I have a huge fear of finding emergencies and must admit to passing William to Paul and being the one to get help when he was in respiritory arrest with septic shock!). Apart from yellow liquid absolutely everywhere in a most spectacular morning explosions all was normal. We had a few bloody streaks and some discomfort during the morning but not so much as to be more of a worry than something that needs to be discussed at tomorrow's clinic appointment. The nurse specialist called make sure all was OK but we were able to leave it to tomorrow to sort out.

To celebrate maintaining our freedom we had an action packed day. I took William to "Wiggle and Jiggle" a song and action session for toddlers at the library. He absolutely loved it and joined in so much. I was amazed how quickly he picked things up. He knows every action to his favourites, the wheels on the bus, wind the bobbin up and all 5 versus of row your boat. He was so good at staying on the carpet and joining in.

The sun was out at last and when Paul arrived home from a rehearsal in the afternoon, he decided to get out the little paddling pool (filled with buckets) and road test the dry suit at last! William hasn't been in anything more than an inch of water since September and has never yet been swimming so at first he was a little apprehensive. He soon relaxed and really started to enjoy himself. It was lovely to see him in there. He could splash and splash without the fear that it would wet his dressing too much. He had such a great time. Now he will be able to go swimming and paddle in the sea and really enjoy the water rather then have me quickly take him out of the bath the minute he starts to enjoy it. Thanks so much to REACT for buying this for us. I can't wait to take him into the pool at the hospice next weekend.

Busy Day

A very interesting day! This morning I had an interview for another part time job I can do from home..and the good news is...I got it! Will post more about that tomorrow when it is all formalised. As I had William cover for the day I then went to meet the lovely Emily AKA Angel. We chatted for hours (boy can we both talk!) and I couldn't believe that I had never actually met her before. I had such a chilled day and had no idea that poor Wills was having yet another little problem. The poor thing has been really uncomfortable this afternoon and has been passing fresh blood. I wasn't told over the phone as Paul had made all the phone calls and taken him to the hospital for blood tests and there was nothing more I could do...except stress!!! The blood clotting looks OK but we have to contact the Chelsea and Westminster if he passes anymore so we have another night and morning hoping all is OK and wondering if we will be back in hospital again. Both Paul and I are pretty nervous and squemish about what we might find so are both pleading with the other to change him at the moment ;-)

Hooray!!!

Just a brief post today to say that Dad got the all clear at his appointment!!! We were all expecting the worse but it turns out there is just a little thickening at the site of his previous op that the radiologist thought could have been cancer cells. The consultant really doesn't think so but says, even if it is, it will be very easy to treat. They will check it out in a few weeks. I am so relieved!!!

William is a bit better too. He is still very wheezy and has a cough that is frequently turning him purple but is a little more energetic. His bloods today showed an Hb of 13! Fantastic - thanks to his transfusion of packed red cells last week. His liver enzymes are high though, which was not a surprise as his liver is currently huge and bulging. This shows his liver is getting a bit cross with the combination of TPN and a chest infection. All this takes its toll in the long term so we could really do with as little extra illness as possible. He is also breaking out in the most horrible red and angry excema in huge patches. I think he is a little bit run down at the moment - still smiling though as always!

It was all yellow...

OK, so usually you see a lovely cute picture of William here. This time I have put a delightful picture of 6 baby grows heavily stained in the yellow stuff that pours out of William's bottom, gastrostomy and jejenostomy. Last night was particularly spectacular and this is the result - yep, one night! and there are 7 sheets too! I took this picture in case I have to convince our water company that we have a medical need for a large water usage if the shortages get worse. I put it here because one key purpose of this blog is to show people what it is like to have a severe intestinal condition and this is a large part of it!
Poor William's bottom is stained yellow - I often sing the Coldplay song to him. It will become even more relevant when he finally goes yellow with jaundice - well, you have to find the funny side don't you! Not that it is easy to find when you have changed a child and made his bed every couple of hours all night. Luckily for me, our lovely night respite nurse, Margaret, was here last night and usually this only happens about 3 times a night.

It was very moving in church this morning. There had been a funeral on Friday for a young girl who suddenly died due to a huge epileptic fit. Her Mum is a regular member of the church and so a wonderful display of her art work and horse riding rosettes had been left at the front where it had been for her funeral. I was so moved, she was so talented. I thought this was such a lovely idea and certainly something that will go into the notebook I have in case we ever suddenly find ourself in that position. Wills has some lovely art work and photos. I have to capture every moment of his life and have a special scrap book. It will be lovely to have whatever happens as this period of our life will always be worth looking back on as we have gone through, and are still going through, so much. Still, I do have my notebook because if anything does happen I want to be able to remember all the thoughts I had to make it a really special day and I know the grief and shock would stop me being able to do so. Oh and yes, "Yellow" is down there - a bit of a joke to myself to make me smile if I am ever in the situation to need to refer to it!

Unsettling times

First I want to say a huge thank-you to Emily, Emma and Shadow who are alwaus so quick with words of encouragement when things are very good or very bad (we hardly seem to get an inbetween! Maybe because we are so busy making the best of the good times). It makes so much difference knowing people are thinking of us and rooting foir us. Willam says a special hello to Nayan - thank-you for thinking about him.
Well, we made it through the day at home by the skin of our teeth! William is still pretty poorly and is coughing and choking himself purple. He is so congested. It is a very long time since I have seen him like this. All the extra secretions that are swallowed are upsetting his tummy as he can't tolerate anything in it. He doesn't really know what to do with himself but has still spent a lot of the day smiling and laughing as usual! He looks nice and pink though as you can see. This is due to the blood transfusion he had on Thursday and is a rare sight as his Hb slowly drops between transfusions and tends to plumet when he is unwell. I think he is a bit flushed and flustered too but it makes him look well!
Paul is away singing a gig in Somerset today and I hate it when he is away and William is unwell because there is no-one to ask what they think of him and how he is. I have to make the call alone and sometimes wonder if I want him home so much the doctors let him home when he is still a little bit too under the weather.
I was catching up with the CF Trust message boards today and learned that a man with a young family has died and another young lad is very sick and his mother is terrified. I don't know either of them but it really upset me. When you are in similar positions you can physically feel the pain they must be feeling. Of course, this kind of news also brings your own agonising fears that you fight so hard to keep at the back of your to the forefront of your thinking.
This is an axious weekend for our family for another reason as well. My Dad had a huge cancer op 2 years ago. He has recently had a lymph node enlarged and had a full body scan 4 weeks ago. On Friday, he had a call to say his results have been discussed at a multi-disciplinary meeting and he has to come in and see his consultant first thing Monday morning. It sounds very ominous. I don't feel stressed though. I don't know if this is because I have been so stressed over the last year that I am spent or because I have learned now that some things just can't be changed and you just have to be positive and make the best of what you do have. I hope it is the latter. It is probably a combination of the two!

The bubble has finally burst!!!

Well, it had to happen! We were having such a lovely run with William. He has had a cold/hayfever for weeks but woke up on Tuesday morning very very chesty and out of puff. Through the day he sounded dreadful but not too worryingly so. We went to the GP and got some antibiotics. However, an hour or so after falling asleep he was lying looking very pale and breathing at a rate of 60 breaths a minute. His temperature had hit the magic, potential line infection, 38 degrees so off the A and E we went. One of our friends looked after the girls for the night which was fantastic. It turned out that William has pneumonia on the right side. He also had a very low Hb level (anaemic) so needed a blood transfusion. This seems to happen when he is unwell. The initial doctor decided to ignore Chelsea and Westminster's protocol that IV antibiotics have to be started if William has a temperature 38 or above and stopped after 48 hours if blood cultures stay negative. Instead she gave him oral ones for his chest. Unfortunately, these really upset his guts with the result of 400mls of bottle green gastric aspirate over night, continuous windy watery poos, wretching and tummy pain and distention. We didn't know what would happen with the addition of the antibiotics as the volume into his system would be much more than the meds he has at the moment. It is a bit worrying that it was no unpleasant as it doesn't look good for the chance of reintroducing food for the time being. The next morning they started the IVs and he responded well. His blood cultures came back Ok this morning so we are now home but on IV antibiotics for his chest. It is a shame because his chest had been good following his Nissen op for reflux but this is the second lot of IV antibiotics for his chest in 2 months and his X-ray showed "changes" on the right. I hope he stays well enought to be at home but he is very chesty and wheezy and a bit grey and blue around his mouth and face. We are having to keep him on constant drainage at the moment as his poor tummy is really struggling with the coughing. It may be that he aspirated on the "snotty" cold secretions and that caused an aspiration pneumonia. We may have to go back to suction to keep his upper airways clear of normal baby secretions. We'll see!

It was most unpleasant walking into the house as we had to drop everything and take him and the remains of 4 day old chicken thighs and rice were still on plates! Lovely! At least it hadn't been too hot. Paul had been busy with Glyndeborne Opera and the girls staying with friends so none of us had been home. One of us has to be with William all the time when he is in, well you do in any case with such a young child, but when he is in the local hospital we really have to stay on the ball as many staff are not so experienced with TPN etc as the specialist centres. I am really looking forward to a nice bottle of wine, a hot bath and an early night in my own bed!